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Pediatrics
Diagnosis
Brain
Pathology-Based Diagnoses
Vascular Malformations
CVMs Without AV Shunting
Cavernous Malformation
Cavernous Malformation
Luke L. Linscott, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Cavernoma, cavernous hemangioma, cavernous angioma, angiographically occult vascular malformation

              • Definitions

                • Cavernous malformation (CM): Benign vascular lesion arising in CNS
                • Consists of dilated sinusoids containing blood products of various ages & lined by thin, immature walls

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Angiographic Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Goyal P et al: Pediatric congenital cerebrovascular anomalies. J Neuroimaging. 29(2):165-81, 2019
                                              2. Neu MA et al: Susceptibility-weighted magnetic resonance imaging of cerebrovascular sequelae after radiotherapy for pediatric brain tumors. Radiother Oncol. 127(2):280-6, 2018
                                              3. Spiegler S et al: Cerebral cavernous malformations: an update on prevalence, molecular genetic analyses, and genetic counselling. Mol Syndromol. 9(2):60-9, 2018
                                              4. Brinjikji W et al: Prevalence of cerebral cavernous malformations associated with developmental venous anomalies increases with age. Childs Nerv Syst. 33(9):1539-43, 2017
                                              5. Young A et al: Sensitivity of susceptibility-weighted imaging in detecting developmental venous anomalies and associated cavernomas and microhemorrhages in children. Neuroradiology. 59(8):797-802, 2017
                                              6. Choquet H et al: Genetics of cerebral cavernous malformations: current status and future prospects. J Neurosurg Sci. 59(3):211-20, 2015
                                              7. Gross BA et al: The natural history of cerebral cavernous malformations in children. J Neurosurg Pediatr. 1-6, 2015
                                              8. Horne MA et al: Clinical course of untreated cerebral cavernous malformations: a meta-analysis of individual patient data. Lancet Neurol.15(2):166-73, 2015
                                              9. Kosnik-Infinger L et al: Management of cerebral cavernous malformations in the pediatric population: a literature review and case illustrations. J Neurosurg Sci. 59(3):283-94, 2015
                                              10. Nikoubashman O et al: Prospective hemorrhage rates of cerebral cavernous malformations in children and adolescents based on MRI appearance. AJNR Am J Neuroradiol. 36(11):2177-83, 2015
                                              11. Di Giannatale A et al: Natural history of cavernous malformations in children with brain tumors treated with radiotherapy and chemotherapy. J Neurooncol. 117(2):311-20, 2014
                                              12. Mikati AG et al: Dynamic permeability and quantitative susceptibility: related imaging biomarkers in cerebral cavernous malformations. Stroke. 45(2):598-601, 2014
                                              13. Nagy G et al: Stereotactic radiosurgery of intracranial cavernous malformations. Neurosurg Clin N Am. 24(4):575-89, 2013
                                              14. Al-Holou WN et al: Natural history and imaging prevalence of cavernous malformations in children and young adults. J Neurosurg Pediatr. 9(2):198-205, 2012
                                              15. Alexiou GA et al: Treatment options for central nervous system cavernous malformations in children. Eur J Paediatr Neurol. 15(3):281, 2011
                                              16. Chang EF et al: Supratentorial cavernous malformations in eloquent and deep locations: surgical approaches and outcomes. Clinical article. J Neurosurg. 114(3):814-27, 2011
                                              17. Ozgen B et al: Radiological features of childhood giant cavernous malformations. Neuroradiology. 53(4):283-9, 2011
                                              18. Abla AA et al: Cavernous malformations of the brainstem presenting in childhood: surgical experience in 40 patients. Neurosurgery. 67(6):1589-98; discussion 1598-9, 2010
                                              19. Smith ER et al: Cavernous malformations. Neurosurg Clin N Am. 21(3):483-90, 2010
                                              20. Chen L et al: Apoptotic functions of PDCD10/CCM3, the gene mutated in cerebral cavernous malformation 3. Stroke. 40(4):1474-81, 2009
                                              21. Shenkar R et al: Advanced magnetic resonance imaging of cerebral cavernous malformations: part I. High-field imaging of excised human lesions. Neurosurgery. 63(4):782-9; discussion 789, 2008
                                              22. Shenkar R et al: Advanced magnetic resonance imaging of cerebral cavernous malformations: part II. Imaging of lesions in murine models. Neurosurgery. 63(4):790-7; discussion 797-8, 2008
                                              23. Yun TJ et al: A T1 hyperintense perilesional signal aids in the differentiation of a cavernous angioma from other hemorrhagic masses. AJNR Am J Neuroradiol. 29(3):494-500, 2008
                                              24. Reich P et al: Molecular genetic investigations in the CCM1 gene in sporadic cerebral cavernomas. Neurology. 60(7):1135-8, 2003
                                              25. Kehrer-Sawatzki H et al: Mutation and expression analysis of the KRIT1 gene associated with cerebral cavernous malformations (CCM1). Acta Neuropathol (Berl). 104(3):231-40, 2002
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    • Pathology

                                                      • Clinical Issues

                                                        • Diagnostic Checklist

                                                          TERMINOLOGY

                                                          • Synonyms

                                                            • Cavernoma, cavernous hemangioma, cavernous angioma, angiographically occult vascular malformation

                                                          • Definitions

                                                            • Cavernous malformation (CM): Benign vascular lesion arising in CNS
                                                            • Consists of dilated sinusoids containing blood products of various ages & lined by thin, immature walls

                                                          IMAGING

                                                          • General Features

                                                            • CT Findings

                                                              • MR Findings

                                                                • Angiographic Findings

                                                                  • Imaging Recommendations

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Staging, Grading, & Classification

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Goyal P et al: Pediatric congenital cerebrovascular anomalies. J Neuroimaging. 29(2):165-81, 2019
                                                                                          2. Neu MA et al: Susceptibility-weighted magnetic resonance imaging of cerebrovascular sequelae after radiotherapy for pediatric brain tumors. Radiother Oncol. 127(2):280-6, 2018
                                                                                          3. Spiegler S et al: Cerebral cavernous malformations: an update on prevalence, molecular genetic analyses, and genetic counselling. Mol Syndromol. 9(2):60-9, 2018
                                                                                          4. Brinjikji W et al: Prevalence of cerebral cavernous malformations associated with developmental venous anomalies increases with age. Childs Nerv Syst. 33(9):1539-43, 2017
                                                                                          5. Young A et al: Sensitivity of susceptibility-weighted imaging in detecting developmental venous anomalies and associated cavernomas and microhemorrhages in children. Neuroradiology. 59(8):797-802, 2017
                                                                                          6. Choquet H et al: Genetics of cerebral cavernous malformations: current status and future prospects. J Neurosurg Sci. 59(3):211-20, 2015
                                                                                          7. Gross BA et al: The natural history of cerebral cavernous malformations in children. J Neurosurg Pediatr. 1-6, 2015
                                                                                          8. Horne MA et al: Clinical course of untreated cerebral cavernous malformations: a meta-analysis of individual patient data. Lancet Neurol.15(2):166-73, 2015
                                                                                          9. Kosnik-Infinger L et al: Management of cerebral cavernous malformations in the pediatric population: a literature review and case illustrations. J Neurosurg Sci. 59(3):283-94, 2015
                                                                                          10. Nikoubashman O et al: Prospective hemorrhage rates of cerebral cavernous malformations in children and adolescents based on MRI appearance. AJNR Am J Neuroradiol. 36(11):2177-83, 2015
                                                                                          11. Di Giannatale A et al: Natural history of cavernous malformations in children with brain tumors treated with radiotherapy and chemotherapy. J Neurooncol. 117(2):311-20, 2014
                                                                                          12. Mikati AG et al: Dynamic permeability and quantitative susceptibility: related imaging biomarkers in cerebral cavernous malformations. Stroke. 45(2):598-601, 2014
                                                                                          13. Nagy G et al: Stereotactic radiosurgery of intracranial cavernous malformations. Neurosurg Clin N Am. 24(4):575-89, 2013
                                                                                          14. Al-Holou WN et al: Natural history and imaging prevalence of cavernous malformations in children and young adults. J Neurosurg Pediatr. 9(2):198-205, 2012
                                                                                          15. Alexiou GA et al: Treatment options for central nervous system cavernous malformations in children. Eur J Paediatr Neurol. 15(3):281, 2011
                                                                                          16. Chang EF et al: Supratentorial cavernous malformations in eloquent and deep locations: surgical approaches and outcomes. Clinical article. J Neurosurg. 114(3):814-27, 2011
                                                                                          17. Ozgen B et al: Radiological features of childhood giant cavernous malformations. Neuroradiology. 53(4):283-9, 2011
                                                                                          18. Abla AA et al: Cavernous malformations of the brainstem presenting in childhood: surgical experience in 40 patients. Neurosurgery. 67(6):1589-98; discussion 1598-9, 2010
                                                                                          19. Smith ER et al: Cavernous malformations. Neurosurg Clin N Am. 21(3):483-90, 2010
                                                                                          20. Chen L et al: Apoptotic functions of PDCD10/CCM3, the gene mutated in cerebral cavernous malformation 3. Stroke. 40(4):1474-81, 2009
                                                                                          21. Shenkar R et al: Advanced magnetic resonance imaging of cerebral cavernous malformations: part I. High-field imaging of excised human lesions. Neurosurgery. 63(4):782-9; discussion 789, 2008
                                                                                          22. Shenkar R et al: Advanced magnetic resonance imaging of cerebral cavernous malformations: part II. Imaging of lesions in murine models. Neurosurgery. 63(4):790-7; discussion 797-8, 2008
                                                                                          23. Yun TJ et al: A T1 hyperintense perilesional signal aids in the differentiation of a cavernous angioma from other hemorrhagic masses. AJNR Am J Neuroradiol. 29(3):494-500, 2008
                                                                                          24. Reich P et al: Molecular genetic investigations in the CCM1 gene in sporadic cerebral cavernomas. Neurology. 60(7):1135-8, 2003
                                                                                          25. Kehrer-Sawatzki H et al: Mutation and expression analysis of the KRIT1 gene associated with cerebral cavernous malformations (CCM1). Acta Neuropathol (Berl). 104(3):231-40, 2002
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